Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0034186 (pyelonephritis)
 6,144 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 46-yr-old female with chronic pyelonephritis was found to lack complement (C) activity by the use of hemolytic screen assays in agarose gels. These assays also revealed a propensity of patient serum to form an activated complex of the fifth and sixth components of C, C56. Each of the C component hemolytic activities was present in normal or elevated amounts with the exception of C7, which was undetectable; addition of purified C7 led to the restoration of hemolytic activity. C-dependent phagocytosis, immune adherence, and neutrophil chemotaxis were normal. Family studies demonstrated that the defect was transmitted as an autosomal codominant apparently not linked with alleles at the HLA-A or HLA-B loci. Persisting C56 was readily formed in this as compared to normal serum upon incubation with multiple C activators including zymosan, inulin, immune complexes, heat-aggregated human gamma globulin, endotoxin, and agarose. A heat-stable (56 degrees C, 30 min) activity which consumed C7 with time-and temperature-dependent kinetics was detected in plasma and serum, and seemed to be similar to a "C7 inactivator" previously described in another C7-deficient individual. However, this activity was found to have properties identical to those of C56 during low ionic strength precipitation and chromatography on Sephadex G-200, to be specifically removed upon passage through an anti-C5 immunoadsorbent column, and to be associated with a small amount of C56, suggesting that it represents an expression of small amounts of C56 rather than a new C-inhibitory activity. Thus, an individual with chronic nephritis lacking C7 is reported; the utility of a hemolytic screen assay in agarose plates for the detection of such patients is emphasized; persisting C56 is shown readily to be formed in this serum; and the presence of C7-consuming activity which is associated with and in all likelihood attributable to C56 is shown.
 ...
PMID:Inherited deficiency of the seventh component of complement associated with nephritis. Propensity to formation of C56 and related C7-consuming activity. 35 Sep 2

1. The use of CsA in cadaver donor transplants has apparently overcome the effect of original disease one-year graft survival rates. Only SLE patients had lower than average graft survival rates in CsA-treated, first transplants. 2. Since 1970, the proportion of diabetics transplanted has increased tenfold. The proportions of transplants for glomerulonephritis and pyelonephritis have decreased over the years. 3. A beneficial effect of pretransplant blood transfusions, was observed in almost all of the disease groups. 4. HLA matching, particularly for HLA-B, DR antigens, has resulted in increased graft survival rates in the major disease categories. Small numbers of zero mismatched grafts prevented a more detailed analysis. 5. Whereas CsA consistently enhanced graft survival rates for first cadaver transplants, this drug had a much smaller effect in living donor transplants. A 14% increase was seen in cadaver donor transplants due to CsA, compared to 2% (siblings), 1% (parent), and 4% (child) for the living donor grafts.
 ...
PMID:Original disease of the recipient. 315 36